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https://www.readbyqxmd.com/read/28917203/cognitive-behavioral-correlates-of-proxy-reports-on-cognitive-capabilities-in-pediatric-patients-with-epilepsy
#1
Christoph Helmstaedter, Renata Vaz Pandolfo, Christian Hoppe, Juri-Alexander Witt
PURPOSE: To validate the Cognitive Problems in Children and Adolescents Questionnaire (KOPKIJ, German: Kognitive Probleme bei Kindern und Jugendlichen), a proxy report measure for recognizing cognitive problems in pediatric patients with epilepsy. METHODS: Anonymized data sets from 279 pediatric epilepsy patients were standardized in regard to the KOPKIJ results of 352 healthy children and adolescents. The KOPKIJ was related to objective routine neuropsychological assessment (NPY), and to two subjective measures, the Child Behavior Checklist questionnaire (CBCL), a proxy rating by the parents, and a questionnaire for self-perceived health-related quality of life in children and adolescents (KINDL)...
September 6, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28914199/clinical-applications-of-intravenous-immunoglobulins-in-child-neurology
#2
Maria Gogou, Efimia Papadopoulou-Alataki, Martha Spilioti, Sofia Alataki, Athanasios Evangeliou
BACKGROUND: While there are guidelines for the use of intravenous immunoglobulins in children with Guillain-Barre syndrome and myasthenia gravis based on high-level evidence studies, data is fewer for the majority of neurologic disorders in this age group. Neuronal antibodies are detected in children with seizures of autoimmune etiology. Intravenous immunoglobulins with their broad immunomodulatory mechanism of action could be ideally effective in different forms of immune-dysregulated intractable epilepsies such as autoimmune epilepsy and autoimmune Rasmussen encephalitis...
September 15, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28901536/validation-of-the-pedsql-epilepsy-module-a-pediatric-epilepsy-specific-health-related-quality-of-life-measure
#3
Avani C Modi, Katherine F Junger, Constance A Mara, Tanja Kellermann, Lauren Barrett, Janelle Wagner, Grace A Mucci, Laurie Bailey, Dace Almane, Shanna M Guilfoyle, Lauryn Urso, Brooke Hater, Heather Hustzi, Gigi Smith, Bruce Herrmann, M Scott Perry, Mary Zupanc, James W Varni
OBJECTIVE: To validate a brief and reliable epilepsy-specific, health-related quality of life (HRQOL) measure in children with various seizure types, treatments, and demographic characteristics. METHODS: This national validation study was conducted across five epilepsy centers in the United States. Youth 5-18 years and caregivers of youth 2-18 years diagnosed with epilepsy completed the PedsQL Epilepsy Module and additional questionnaires to establish reliability and validity of the epilepsy-specific HRQOL instrument...
September 13, 2017: Epilepsia
https://www.readbyqxmd.com/read/28888970/glial-overexpression-of-dube3a-causes-seizures-and-synaptic-impairments-in-drosophila-concomitant-with-down-regulation-of-the-na-k-pump-atp%C3%AE
#4
Kevin A Hope, Mark S LeDoux, Lawrence T Reiter
Duplication 15q syndrome (Dup15q) is an autism-associated disorder co-incident with high rates of pediatric epilepsy. Additional copies of the E3 ubiquitin ligase UBE3A are thought to cause Dup15q phenotypes, yet models overexpressing UBE3A in neurons have not recapitulated the epilepsy phenotype. We show that Drosophila endogenously expresses Dube3a (fly UBE3A homolog) in glial cells and neurons, prompting an investigation into the consequences of glial Dube3a overexpression. Here we expand on previous work showing that the Na(+)/K(+) pump ATPα is a direct ubiquitin ligase substrate of Dube3a...
September 6, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28880998/impaired-bone-and-muscle-development-in-young-people-treated-with-antiepileptic-drugs
#5
Peter J Simm, Sebastian Seah, Alex Gorelik, Lauren Gilbert, Jenning Nuguid, George A Werther, Mark T Mackay, Jeremy L Freeman, Sandra J Petty, John D Wark
OBJECTIVE: Antiepileptic drugs (AEDs) are associated with reduced bone density, balance impairment, and increased fracture risk in adults. However, pediatric data are limited. Therefore, we aimed to examine bone, muscle, and balance outcomes in young patients taking AEDs. METHODS: We undertook a case-control study utilizing an AED exposure-discordant matched-pair approach. Subjects were aged 5-18 years with at least 12 months of AED exposure. Pairs were twins, nontwin siblings and first cousins, sex- and age-matched (to within 2 years), allowing for greater power than with unrelated control subjects...
September 7, 2017: Epilepsia
https://www.readbyqxmd.com/read/28873364/refractory-spasms-of-focal-onset-a-potentially-curable-disease-that-should-lead-to-rapid-surgical-evaluation
#6
Mathilde Chipaux, Georg Dorfmüller, Martine Fohlen, Nathalie Dorison, Marie-Astrid Metten, Olivier Delalande, Sarah Ferrand-Sorbets, Delphine Taussig
PURPOSE: Infantile spasms (IS) can occur as the only seizure type in children with surgically amenable epilepsies. Although early surgery has shown positive effects, little is known regarding outcomes. METHODS: We retrospectively reviewed all children with IS referred to our tertiary center between 2002 and 2014 and try to define factors of outcome. RESULTS: Sixty-eight children with focal onset seizures were referred: twenty children with a hemispheric implication and 48 with one or more lobes involved...
August 31, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28866334/increasing-volume-and-complexity-of-pediatric-epilepsy-surgery-with-stable-seizure-outcome-between-2008-and-2014-a-nationwide-multicenter-study
#7
Carmen Barba, Nicola Specchio, Renzo Guerrini, Laura Tassi, Salvatore De Masi, Francesco Cardinale, Simona Pellacani, Luca De Palma, Domenica Battaglia, Gianpiero Tamburrini, Giuseppe Didato, Elena Freri, Alessandro Consales, Paolo Nozza, Nelia Zamponi, Elisabetta Cesaroni, Giancarlo Di Gennaro, Vincenzo Esposito, Marco Giulioni, Paolo Tinuper, Gabriella Colicchio, Raffaele Rocchi, Guido Rubboli, Flavio Giordano, Giorgio Lo Russo, Carlo Efisio Marras, Massimo Cossu
OBJECTIVE: The objective of the study was to assess common practice in pediatric epilepsy surgery in Italy between 2008 and 2014. METHODS: A survey was conducted among nine Italian epilepsy surgery centers to collect information on presurgical and postsurgical evaluation protocols, volumes and types of surgical interventions, and etiologies and seizure outcomes in pediatric epilepsy surgery between 2008 and 2014. RESULTS: Retrospective data on 527 surgical procedures were collected...
August 31, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28865255/parent-use-of-cannabis-for-intractable-pediatric-epilepsy-everyday-empiricism-and-the-boundaries-of-scientific-medicine
#8
Elisa J Sobo
Cannabis is an increasingly sought-after remedy for US children with intractable (biomedically uncontrollable) epilepsy. However, like other complementary-alternative medicine (CAM) modalities, and particularly as a federally illegal, stigmatized substance, it is unsanctioned by mainstream medicine. Parents are largely on their own when it comes to learning about, procuring, dispensing, and monitoring treatments. Exploring how they manage is crucial to better assist them. Moreover, it can illuminate how 'research' done on the ground by laypeople variously disrupts and reinforces lay-expert and science-non-science divides...
August 5, 2017: Social Science & Medicine
https://www.readbyqxmd.com/read/28860215/american-society-of-functional-neuroradiology-recommended-fmri-paradigm-algorithms-for-presurgical-language-assessment
#9
D F Black, B Vachha, A Mian, S H Faro, M Maheshwari, H I Sair, J R Petrella, J J Pillai, K Welker
INTRODUCTION: Functional MR imaging is increasingly being used for presurgical language assessment in the treatment of patients with brain tumors, epilepsy, vascular malformations, and other conditions. The inherent complexity of fMRI, which includes numerous processing steps and selective analyses, is compounded by institution-unique approaches to patient training, paradigm choice, and an eclectic array of postprocessing options from various vendors. Consequently, institutions perform fMRI in such markedly different manners that data sharing, comparison, and generalization of results are difficult...
August 31, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28846756/prevalence-of-pathogenic-copy-number-variation-in-adults-with-pediatric-onset-epilepsy-and-intellectual-disability
#10
Felippe Borlot, Brigid M Regan, Anne S Bassett, D James Stavropoulos, Danielle M Andrade
Importance: Copy number variation (CNV) is an important cause of neuropsychiatric disorders. Little is known about the role of CNV in adults with epilepsy and intellectual disability. Objectives: To evaluate the prevalence of pathogenic CNVs and identify possible candidate CNVs and genes in patients with epilepsy and intellectual disability. Design, Setting, and Participants: In this cross-sectional study, genome-wide microarray was used to evaluate a cohort of 143 adults with unexplained childhood-onset epilepsy and intellectual disability who were recruited from the Toronto Western Hospital epilepsy outpatient clinic from January 1, 2012, through December 31, 2014...
August 28, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28844439/comorbidities-and-risk-factors-associated-with-newly-diagnosed-epilepsy-in-the-u-s-pediatric-population
#11
Ahyuda Oh, David J Thurman, Hyunmi Kim
Neurobehavioral comorbidities can be related to underlying etiology of epilepsy, epilepsy itself, and adverse effects of antiepileptic drugs. We examined the relationship between neurobehavioral comorbidities and putative risk factors for epilepsy in children with newly diagnosed epilepsy. We conducted a retrospective analysis of children aged ≤18years in 50 states and the District of Columbia, using the Truven Health MarketScan® commercial claims and encounters database from January 1, 2009 to December 31, 2013...
August 22, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28843212/the-influence-of-endophenotypic-disease-specific-and-environmental-variables-on-the-expression-of-anxiety-in-pediatric-epilepsy
#12
William A Schraegle, Jeffrey B Titus
Children and adolescents with epilepsy often show higher rates of anxiety, which carries an increased risk for reduced health-related quality of life (HRQOL). The current study assessed the role of parental psychiatric history (i.e., anxiety, depression, and/or bipolar disorder) on the rate of anxiety features in youth seen in a tertiary epilepsy clinic. Data included parental ratings on the Behavior Assessment System for Children (BASC-2) and the Quality of Life in Childhood Epilepsy (QOLCE) questionnaire for 180 children and adolescents (mean age=11...
August 23, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28843210/systematic-review-of-unmet-healthcare-needs-in-patients-with-epilepsy
#13
REVIEW
Mayuri Mahendran, Kathy N Speechley, Elysa Widjaja
OBJECTIVE: Patients with epilepsy (PWE) are more likely to have unmet healthcare needs than the general population. This systematic review assessed the reasons for unmet needs in PWE. METHODS: Medline, Embase, PsycINFO, Cochrane, and Web of Science databases were searched using keywords relating to unmet healthcare needs, treatment barriers, and access to care. The search included all countries, adult and pediatric populations, survey and qualitative studies, but excluded non-English articles and articles published before 2001...
August 23, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28837898/patterns-of-intrahemispheric-propagation-in-pediatric-photoparoxysmal-response
#14
Tymon Skadorwa, Jolanta Strzelecka
PURPOSE: The photoparoxysmal response (PPR) is the most important EEG indication of photosensitivity (PS). It may be elicited by intermittent photic stimulation (IPS). PS mostly affects young individuals, with adolescent females at greatest risk. The diagnosis of PS is based on visual EEG assessment. To date, no objective test of PS has been established. METHOD: Here we describe 89 individuals of both sexes, aged 5-18 years, epileptic and non-epileptic, in whom a PPR was elicited by IPS during a standard EEG procedure...
August 12, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28831924/management-of-antiepileptic-treatment-after-epilepsy-surgery-practices-and-problems
#15
Guido Rubboli, Anne Sabers, Peter Uldall, Kristina Malmgren
BACKGROUND: Although epilepsy surgery is a recognized treatment option for drug-resistant epilepsies since several decades, the management of antiepileptic drugs (AEDs) after successful surgery still remains one of the most difficult and unsolved therapeutic challenges. Indeed, no systematic controlled trials have been specifically conducted so far and no consensus or standardized guidelines regarding postsurgical drug discontinuation policy and procedures are available. METHODS: In this paper, we aim to provide an updated overview on the the present knowledge on this topic, which is based mainly on retrospective studies reporting practices used in individual centers...
August 22, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28821006/adherence-to-antiepileptic-drugs-in-children-and-adolescents-a-retrospective-study-in-primary-care-settings-in-germany
#16
Louis Jacob, Hajo M Hamer, Karel Kostev
OBJECTIVE: The goal of this study was to analyze adherence to antiepileptic drugs (AED) in children and adolescents treated in pediatric practices in Germany. METHODS: The present study included patients aged between 2 and 17years who were diagnosed with epilepsy (ICD-10: G40) and had received at least two prescriptions of AED between January 2006 and December 2015 in 243 pediatric practices in Germany. The medication possession ratio (MPR) was used to estimate adherence, and patients with a MPR greater than 80% were considered adherent...
August 15, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28808577/transition-from-intravenous-to-enteral-ketamine-for-treatment-of-nonconvulsive-status-epilepticus
#17
Michael A Pizzi, Prasuna Kamireddi, William O Tatum, Jerry J Shih, Daniel A Jackson, William D Freeman
BACKGROUND: Nonconvulsive status epilepticus (NCSE) is a diagnosis that is often challenging and one that may progress to refractory NCSE. Ketamine is a noncompetitive N-methyl-d-aspartate antagonist that increasingly has been used to treat refractory status epilepticus. Current Neurocritical Care Society guidelines recommend intravenous (IV) ketamine infusion as an alternative treatment for refractory status epilepticus in adults. On the other hand, enteral ketamine use in NCSE has been reported in only 6 cases (1 adult and 5 pediatric) in the literature to date...
2017: Journal of Intensive Care
https://www.readbyqxmd.com/read/28807611/initial-treatment-for-nonsyndromic-early-life-epilepsy-an-unexpected-consensus
#18
Renée A Shellhaas, Anne T Berg, Zachary M Grinspan, Courtney J Wusthoff, John J Millichap, Tobias Loddenkemper, Jason Coryell, Russell P Saneto, Catherine J Chu, Sucheta M Joshi, Joseph E Sullivan, Kelly G Knupp, Eric H Kossoff, Cynthia Keator, Elaine C Wirrell, John R Mytinger, Ignacio Valencia, Shavonne Massey, William D Gaillard
OBJECTIVE: There are no evidence-based guidelines on the preferred approach to treating early-life epilepsy. We examined initial therapy selection in a contemporary US cohort of children with newly diagnosed, nonsyndromic, early-life epilepsy (onset before age three years). METHODS: Seventeen pediatric epilepsy centers participated in a prospective cohort study of children with newly diagnosed epilepsy with onset under 36 months of age. Details regarding demographics, seizure types, and initial medication selections were obtained from medical records...
June 27, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28804898/epilepsy-surgery-of-low-grade-epilepsy-associated-neuroepithelial-tumors-a-retrospective-nationwide-italian-study
#19
Marco Giulioni, Gianluca Marucci, Veronica Pelliccia, Francesca Gozzo, Carmen Barba, Giuseppe Didato, Flavio Villani, Giancarlo Di Gennaro, Pier Paolo Quarato, Vincenzo Esposito, Alessandro Consales, Matteo Martinoni, Gianfranco Vornetti, Corrado Zenesini, Carlo Efisio Marras, Nicola Specchio, Luca De Palma, Raffaele Rocchi, Flavio Giordano, Giovanni Tringali, Paolo Nozza, Gabriella Colicchio, Guido Rubboli, Giorgio Lo Russo, Renzo Guerrini, Paolo Tinuper, Francesco Cardinale, Massimo Cossu
OBJECTIVE: To analyze the attitude and results of Italian epilepsy surgery centers in the surgical management of "low grade epilepsy associated neuroepithelial tumors" (LEATs). METHODS: We conducted a retrospective study enrolling 339 consecutive patients with LEATs who underwent surgery between January 2009 and June 2015 at eight Italian epilepsy surgery centers. We compared demographic, clinical, pathologic, and surgical features of patients with favorable (Engel class I) and unfavorable (Engel class II, III, and IV) seizure outcome...
August 14, 2017: Epilepsia
https://www.readbyqxmd.com/read/28802771/dmd-and-west-syndrome
#20
Ruxandra Cardas, Catrinel Iliescu, Nina Butoianu, Andreea Seferian, Svetlana Gataullina, Elena Gargaun, Juliette Nectoux, Thierry Bienvenu, Dana Craiu, Teresa Gidaro, Laurent Servais
Duchenne Muscular Dystrophy (DMD) is the most frequent muscular dystrophy in childhood, with a worldwide incidence of one in 5000 live male births. It is due to mutations in the dystrophin gene leading to absence of full-length dystrophin protein. Central nervous system involvement is well-known in Duchenne Muscular Dystrophy. The multiple dystrophin isoforms expressed in brain have important roles in cerebral development and functioning. The association of Duchenne Muscular Dystrophy with seizures has been reported, and there is a higher prevalence of epilepsy in Duchenne Muscular Dystrophy patients (between 6...
July 19, 2017: Neuromuscular Disorders: NMD
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